Longitudinal Study of Peripapillary Thinning in Sickle Cell Hemoglobinopathies

To determine the rate of retinal nerve fiber layer (RNFL) thinning in patients with sickle cell hemoglobinopathies.

This was a prospective cohort study.

Sixty-seven patients averaging 35.8 ± 11.5 years of age at enrollment with electrophoretically confirmed sickle cell hemoglobinopathies followed by the University of Illinois at Chicago retina clinic for ≥1 year were included. Exclusion criteria included a history of diabetes, uncontrolled hypertension, glaucoma, ocular opacities, other retinopathies, and previous retinal procedures. The optic nerve head RNFL thicknesses were measured with spectral-domain optical coherence tomography (Heidelberg Engineering, Inc) at enrollment and subsequent follow-ups. Linear mixed models were used to estimate rates of thinning.

A total of 122 eyes were followed for 3.8 ± 2.0 years (range 1-8 years). Mean global peripapillary RNFL thickness was 100.9 ± 13.0 μm at baseline. Global peripapillary RNFL thickness decreased at a rate of 0.98 μm per year (95% confidence interval [CI] 0.77-1.19 μm/year). A history of stroke was associated with a faster rate of global RNFL thinning (1.72 ± 0.20 vs 0.79 ± 0.12 μm/year, P < .001), whereas a history of hypertension was associated with a slower rate of thinning (0.33 ± 0.27 vs 1.14 ± 0.12 μm/year, P = .002).

Peripapillary RNFL thinning in patients with sickle cell hemoglobinopathies occurred faster in patients with a history of stroke and slower in patients with controlled hypertension. Future studies will compare these rates to those of healthy age- and race-matched individuals.